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[细胞质包涵体:几种疾病的研究及文献综述]

[Cytoplasmic inclusion bodies: a study in several diseases and a review of the literature].

作者信息

Scola R H, Werneck L C, Franco C R

机构信息

Serviço de Doenças Neuromusculares, Universidade Federal do Paraná, Curitiba.

出版信息

Arq Neuropsiquiatr. 1996 Jun;54(2):245-59. doi: 10.1590/s0004-282x1996000200012.

Abstract

Among 1400 muscle biopsies, we studied 16 cases with rimmed vacuoles, whose histology suggests cytoplasm inclusion bodies. We tried to correlate the clinical, laboratory and histopatological data in order to verify the specificity of cytoplasm inclusion bodies to certain diseases. The creatinekinase was increased in 10 cases. In all cases electromyography was abnormal. Muscle histochemistry revealed myopathy in 5 cases, mixed pattern in 7, denervation in 2 and in 2 cases, inflammatory myopathy. Electron microscopy showed the presence of filaments in 8 cases (nuclear, disseminated in cytoplasm or in the subsarcolemmal region). The patients were classified according to history, heredity, laboratory, electrophysiologic, histochemistry data and electron microscopy: in myositis with inclusion cytoplasmic bodies (4 cases), juvenile spinal muscular atrophy (6 cases), distal myopathies (3 cases), limb-girdle dystrophy (2 cases) and peripheral neuropathy (1 case). We present a revision on the pathogenesis and possible etiology of rimmed vacuoles and their relationship with several diseases.

摘要

在1400例肌肉活检中,我们研究了16例有镶边空泡的病例,其组织学表现提示胞质内包涵体。我们试图将临床、实验室和组织病理学数据相关联,以验证胞质内包涵体对某些疾病的特异性。10例患者肌酸激酶升高。所有病例的肌电图均异常。肌肉组织化学显示5例为肌病,7例为混合模式,2例为去神经支配,2例为炎性肌病。电子显微镜检查显示8例存在细丝(核内、散在胞质或肌膜下区域)。根据病史、遗传、实验室、电生理、组织化学数据和电子显微镜检查对患者进行分类:包涵体肌炎(4例)、青少年脊髓性肌萎缩(6例)、远端肌病(3例)、肢带型肌营养不良(2例)和周围神经病(1例)。我们对镶边空泡的发病机制和可能病因及其与几种疾病的关系进行了综述。

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